Systemic lupus erythematosus During pregnancy

1. Systemic lupus erythematosus
During pregnancy
Aboubakr Elnashar
Benha university, Egypt

2. Incidence
Women > men (ratio 9:1)
During the child-bearing years (ratio 15:1).
Incidence: 1 in 1000 women
Aboubakr Elnashar

3. Clinical features
Systemic connective tissue disease
Heterogeneous: variety of clinical& antibody
patterns.
Other autoimmune disorders: 6%.
Periods of disease activity (flares)& remissions.
Average age at diagnosis: 30 y
Aboubakr Elnashar

4. Commonest clinical feature (90%):
Arthritis:
non­erosive, peripheral, tenderness& swelling.
Other features:
Skin: (80%):
malar rash
Photosensitivity
vasculitic lesions on the "fingertips& nail folds,
Raynaud's phenomenon
Discoid lupus.
Aboubakr Elnashar

5. Serositis:
pleuritis, pericarditis
Renal:
glomeru­lonephritis with proteinuria& cellular casts
Neurological:
psychosis, seizures or chorea.
Haematological:
haemolytic anaemia, thrombocytopenia &
lymphopenia or leukopenia.
Aboubakr Elnashar

6. Pathogenesis
Cause: Un known
Genetic predisposition
Environmental triggers e.g. ultraviolet light or viral
infection.
Immunological:
polyclonal B-cell activation
impaired T-cell regulation of the immune response
failure to remove immune complexes.
Circulating non-organ-specific autoantibodies.
Deposition of immune complexes: vasculitis.
Aboubakr Elnashar

7. Diagnosis
American Rheumatic Association criteria
Many patients have a lupus-like illness without
fulfilling these.
1. CBC:
Normochromic normocytic anaemia
Neutropenia
Thrombocytopenia.
2. ESR:
raised {high immunoglobulin levels}
3. CRP:
Normal
4. 3rd or 4th components of complement:
low or falling: active disease.
Aboubakr Elnashar

8. 5. Auto-antibody
a. The most common:
Antinuclear antibody (ANA): (96%)
Titres do not change with disease activity.
b. The most specific
antibodies to double-stranded DNA (78%) and
Smith (Sm).
Glomerulonephritis occurs more frequently in
women with these antibodies.
c. Other
anti-Ro and anti-La: (30%)
anticardiolipin antibodies (aPLs): (40%).
Aboubakr Elnashar

9. Effect of pregnancy on SLE
Flares:
Increases:
from 40% to 60%.
When:
at any stage of pregnancy or the puerperium
more likely immediately postpartum: little evidence
Prediction:
not possible
more likely if disease has been active within 6 ms of
conception.
Aboubakr Elnashar

10. Diagnosis during pregnancy:
difficult
{many features such as hair loss, oedema, palmar &
facial erythema, fatigue, anaemia, raised ESR &
musculoskeletal pain also occur in normal
pregnancy}.
most commonly involving the skin& joints
Prevention:
prophylactic steroids or routine increases of dose:
no effect & not recommended
Aboubakr Elnashar

11. Women with lupus nephritis
SLE nephropathy may manifest for the first time in
pregnancy.
Risk of deterioration
Moderate renal impairment (serum creatinine 125-
200 umol/I): uncomplicated pregnancies.
High baseline serum creatinine: Inc risk of
deterioration
Aboubakr Elnashar

12. Preconception counseling.
1. Delay pregnancy until at least 6 ms after a lupus
nephritis flare.
2. Prediction of the risks to the woman& fetus:
anti-Ro/La
aPLs
Renal function
Blood pressure
Aboubakr Elnashar

13. Effect of SLE on pregnancy
1. The increased risks
 spontaneous miscarriage
fetal death
pre-eclampsia
preterm delivery
IUGR
 Related to
1. Age, parity
2. anticardiolipin antibodies
3. lupus anticoagulant
4. lupus nephritis
5. hypertension
6. active disease at the time of conception or
7. first presentation of SLE during pregnancy.Aboubakr Elnashar

14. During pregnancy, lupus
improves in a third of women
remains unchanged in a third
worsens in the remaining third.
Clinical condition can worsen or flare without
warning
(Khamashta and colleagues, 1997).
Risk of major morbidity during pregnancy
7%
(Petri, 1998)
Lupus can be life threatening to both the mother
and her fetus-infant.
Aboubakr Elnashar

15. Good pregnancy outcome if:
1. Lupus activity has been quiescent for at least 6
months before conception
2. No active renal involvement manifest by
proteinuria or renal dysfunction
3. Superimposed preeclampsia does not develop
4. No evidence of antiphospholipid antibody
activity.
Aboubakr Elnashar

16. Renal lupus:
increased risk of fetal loss, PET, IUGR
particularly if there is hypertension or proteinuria.
Women in remission, but without hypertension,
renal involvement or aPLs:
Risk of pregnancy loss& PET is not higher than in
the general population.
2. Chorea
very rare complication of pregnancy in women with
SLE or aPLs.
Aboubakr Elnashar

17. Management
Preconception counseling
1. Prediction of the risks to the woman& fetus:
 anti-Ro/La
 aPLs
 Renal
 Blood pressure.
2. Outcome is improved if conception occurs during
disease remission.
Aboubakr Elnashar

18. Multidisciplinary team in combined clinics:
Physicians& obstetricians
Monitor
1. disease activity
2. Fetal growth
3. uterine artery Doppler blood flow at 20-24 w
4. umbilical artery blood flow from 24 w
Aboubakr Elnashar

19. Baseline values in early pregnancy:
1. FBC
2. KFT:
U, creatinine, uric acid, quantify any proteinuria.
3. LFT:
4. anti DNA
5. Complement titres
5. Electrolytes
Serial measurements
at intervals dependent on disease severity
Aboubakr Elnashar

20. Disease flare
Symptoms:
Arthralgia, pleuritic pain, skin rash
 Lab:
A. Urine: Red blood cells or cellular casts
B. Rising anti-DNA antibody titre
C. Fall in complement levels
>25% fall in C3 or C4 suggests active SLE.
N.B. elevation of complement split products,
particularly Ba & Bb, often accompanies flares,
so high ratios of CH50: Ba may differentiate
PET from active lupus.
Aboubakr Elnashar

21. Treatment
Corticosteroids are the drugs of choice.
Aboubakr Elnashar

22. Hydroxychloroquine (Plaquenil)
should be continued {stopping may precipitate
flare}.
For control of hypertension:
Drug of choice: methyldopa
2nd -line agents: nifedipine or hydralazine
Although long-term hydralazine& methyldopa use
may rarely induce a SLE-like syndrome, they
are not contraindicated in SLE.
Aboubakr Elnashar

23. Differentiation of active renal lupus from PET
Difficult
1. The two conditions may be superimposed.
2. Hypertension, proteinuria, thrombocytopenia&
even renal impairment are all features of PET
3. A doubling of baseline proteinuria may be
expected in pregnancy but more than this would be
indicative of either worsening lupus nephritis or
PET}
Aboubakr Elnashar

24. How:
1. Hyperuricaemia & abnormal LFT point more
towards PET.
2. Renal biopsy
The only definitive investigation
Rarely undertaken in pregnancy.
More likely to be appropriate prior to fetal viability
Aboubakr Elnashar

25. TT of active lupus nephritis
Increase oral prednisolone
Pulsed IV methyl predisolone
Azathioprine (Imuran).
Cyclophos­phamide: Rarely used
Aboubakr Elnashar

26. If lupus flare & PET cannot be dd beyond 24-28
w, when the fetus is viable:
Delivery
{cure PET
allow administration of cyclophosphamide}
Aboubakr Elnashar

27. Conclusions
There is an increased rate of flare during
pregnancy.
Disease flares must be actively managed with
corticosteroids.
Adverse pregnancy outcome is related to the
presence of renal involvement, hypertension,
antiphospholipid antibodies& disease activity at
the time of conception.
These factors increase the risks of spontaneous
miscarriage, fetal death, PET, PTL& IUGR.
Aboubakr Elnashar

28. Pregnancy care is best undertaken in combined
clinics allowing close monitoring of disease
activity, fetal growth& well-being.
In Ro-positive mothers
risk of transient neonatal cutaneous lupus: 5%
risk of CHB: 2%.
Aboubakr Elnashar

29. Aboubakr Elnashar
Thanks